This booklet has been developed for individuals attending the Montreal Neurological Hospital and Montreal Neurological Institute Post-Polio Clinic and their families. It is meant as an overview of the current information on the late effects of poliomyelitis. Please ask the Clinic Team if you have additional questions or require clarification of the information presented in this handout.

B. What is Post-Polio Syndrome?

The late effects of poliomyelitis or as it is often called, "Post-Polio Syndrome" (PPS), are a variable set of new symptoms ranging from severe fatigue which may follow moderate activity to generalized joint and muscle pain, muscle cramping and progressive muscle weakness. As well, breathing problems, difficulty sleeping and increased sensitivity to cold may be present. A large percentage (up to 50%) of the individuals who contracted paralytic poliomyelitis earlier in their lives are now experiencing at least some of these symptoms.

C. Possible Causes and Stages

The reason that these symptoms occur in post-polio individuals is poorly understood. However, a number of theories have been proposed as to why these individuals develop problems as time progresses. There are four recognized stages in patients who have recovered from paralytic poliomyelitis:

1. During the first or acute stage of paralytic polio, the polio virus infects the spinal cord and destroys all or a portion of the motor nerve cells which transmit information for muscle control to a particular muscle. Paresis, or partial paralysis results when a portion of motor nerve cells innervating a muscle are destroyed because the link between the spinal cord and muscles is interrupt-ed. If all motor nerve cells to a particular muscle are destroyed, complete paralysis (inabili-ty to contract that muscle) will occur. Normal motor units and the acute stage of para-lytic polio is illustrated in the first two drawings of the Figure. A motor unit is defined as a motor neuron (motor nerve cell) and all the muscle fibers (muscle cells) that it innervates.

2. In the second stage, or recovery phase, healthy motor neurons attempt to compensate for the lost connections by growing sprouts and connecting to the muscle fibers whose nerve cells have been destroyed by the virus. This is known as collateral re-innervation. This is not a very efficient way of activating these muscle fibers, but some or all of the ability to activate the muscle is restored. In addition, the innervated muscle fibers may enlarge through exercise, and further improve the force of muscle contraction. This is why we may see improvement after the acute infection. The third drawing in the Figure illustrates the recovery phase after acute paralytic polio.

3. The third stage is a period of stability which usually lasts several decades. During this stage, patients are stable, both neurologically and functionally. This period is thought to be characterized by a "continuous remodelling process". During this stage, some motor nerve sprouts may degenerate, but new sprouts are formed by neighboring nerve cells with reinnervation of the affected- muscle fibers.

4. The fourth stage is a period of decline. It usually starts approximately 3 to 4 decades after polio infection. The most common symptoms are progressive muscle weakness, fatigue, and cramping. Muscle atrophy (or loss of muscle bulk) may also occur in a smaller propor-tion of patients. These symptoms are identified as PPS. It may be caused by "overwork" and/or the normal ageing process. The surviving motor nerve cells which have been con- trolling many more muscle fibers than normal, may gradually give out due to overwork. A distal degeneration of over-extended nerve cells may occur. In addition, motor nerve cells may be lost due to the natural ageing process. This occurs in all people, whether they have had polio or not. By the time individuals reach their 70's or 80's, there may be a loss of approximately 20-30% of these nerves. Because individuals with polio started with fewer motor nerve cells to innervate their muscles, the natural loss of these nerve cells with ageing will be that much more noticeable. In other words, they will tend to be weaker and more easily fatigued than an unaffected individual. The distal motor nerve cell degeneration with disintegration of sprouts is illustrated in the fourth drawing in the Figure.

D. Incidence and Risk Factors

It appears that as many as 1/4 to 1/2 of polio survivors are experiencing new health problems. Persons who are more likely to experience the late effects of polio are those who had severe acute paralytic poliomyelitis. Other possible contributing factors are a greater functional recovery after acute polio, current age (the normal aging process), length of time since acute polio, recent weight gain, and exercising to the point of muscle pain.

E. Symptoms

1. Fatigue and Muscle Weakness

Fatigue can be so severe in some individuals that they must rest in the middle of the day. The onset of fatigue is often sudden and may be accompanied by a headache, a feeling of weak-ness, hot and cold flashes, and sweating. The phenomenon is often referred to as the "Polio Wall". Most commonly, the Polio Wall is experienced in the mid to late afternoon on a daily basis. The best way of dealing with these symptoms is to take short 15-20 minute periods of complete rest before any symptoms are felt. Another way is to reduce the amount of activity performed during the day. This may help control the occurrence or the severity of the symp-toms.

The symptoms of excessive fatigue and muscle weakness often develop approximately 30-40 years after the initial onset of polio. The progression of muscle weakness appears to involve muscles previously weakened by polio, as well as muscles originally thought to be unaffected by the disease. However, new weakness occurs more frequently in muscles previously affected by paralytic polio. -Most likely, the muscles originally thought to be unaffected by polio had mild involvement or subclinical involvement initially.

2. Pain

Muscle pain may be experienced in various locations such as the back, neck or shoulders. Chronic weight bearing and overuse of unprotected joints may cause stress which often results in pain. Generalized achiness comparable to that felt with the flu may be experienced.

One explanation for this pain is that there may have been injuries to the musculoskeletal system. These may occur in the post-polio population because of the tendency to use a strong part of the body to compensate for a weakness elsewhere. Abnormal postures affect the balance of the body and require greater effort from specific muscles which may result in pain.

It is often reported that pain is worse following vigorous exercise. This should be viewed as a sign that affected muscles have been overused and that perhaps a milder form of exercise is indicated.

Muscle cramping is another indication of muscle overuse. Individuals experiencing this symptom are advised to reduce their level of activity and to rest more often. Another cause for generalized pain is fibromyalgia. This is a disorder characterized by chron-ic, generalized muscle pains of unknown etiology. It is found commonly in a post-polio clinic, and can improve with certain treatments.

Less clearly understood is the pain that follows exposure to cold temperatures. This decrease in limb temperature is often described as a severe burning pain, colour changes, and mottling of the skin. Both the coldness of the skin and the pain tend to be long-lasting. Often, hours of warming are required to relieve the symptoms. Those affected by post-polio symptoms often report that both manual dexterity and muscle strength decrease noticeably with exposure to cold, and become more pronounced with aging. These individuals are advised to be aware of cold temperatures and to dress warmly.

3. Sleep Disturbances

It has been noted that some individuals, especially those who experienced initial respiratory involvement, may experience a change in their sleep patterns. Sleep apnea, which is a brief shortage of oxygen to the brain during sleep, may awaken the individual. The individual may have a morning headache or be unrested on awakening. If sleep apnea is suspected, further testing will need to be performed by a pulmonary specialist.

Poor sleep may also be produced by pain. Rest periods may be required to help alleviate daytime fatigue from poor sleep. These should be taken before the point of feeling tired.

F. Treatment

The aim in polio management is to preserve the healthy motor nerves. Every person experiencing the symptoms should undergo a medical assessment. There is no specific diagnostic test for PPS, therefore it is very important to make sure there are no other causes of the new difficulties. Early recognition, corrective procedures, education, use of assis-tive devices, and occasionally use of medications can help to reduce the symptoms described above. Effective treatment regimens can be prescribed on-an individual basis.

1. Energy Conservation

Saving energy is a learned skill and often requires a change in ways of thinking and life-long habits. Energy conservation allows for the pursuit of leisure activities in balance with activi-ties of daily living. Following the basic principles of energy conservation involves using common sense to find easier and more efficient ways of doing activities. Energy conservation techniques applied to personal care and homemaking tasks increase endurance, maintain strength and muscle power. It is the technique that requires the least amount of effort within a given time that gives the most satisfactory result. An individual's willingness to change is the key to effective energy conservation.

Some of the recommendations for saving energy are:

Analyze how much energy you have in one day. How will you use the energy? What are your priorities and goals? (Be realistic.)

Look at the tasks you do during the day and eliminate those which are not essential.

Take time to rest. Establish a flexible schedule which allows adequate rest periods during the day.

Set up workstations and see that all necessary equipment and supplies are present and easily within reach.

Learn how to pace yourself. Take your time with tasks and rest before you are tired.

Try to perform most tasks sitting down, as sitting requires 50% less energy than standing.

Avoid lifting heavy items and, most importantly, do not take chances with safety.

Remember that you do not always have to change what you do, just the way you do it!

These changes will take some practice but can be invaluable in helping to save energy.

2. Exercise

Muscles affected by the polio virus lack the endurance of normal muscles and thus are prone to early fatigue. Repeated overuse of these muscles, pushing them past their point of fatigue, is presently thought to accelerate muscle weakness after paralytic polio. A symptom of over-fatigue is increased muscle cramping and pain. It is therefore recommended that the polio survivor rest at the first sign of fatigue. Also, in being careful not to overwork "polio" muscles, exercise should be minimal. Generally, the best type of exercise for the polio popula-tion involves gentle stretching (to relieve muscle pain, cramping and maintain or improve joint mobility), conditioning (to increase endurance) and low resistive (to maintain/increase strength) exercises such as swimming, riding a stationary bicycle with little tension, a walking program, a low level strengthening program for specific muscles, or Tai Chi, yoga. Again, to preserve motor units, it is essential to stop exercising (or any activity) and rest at the first sign of fatigue or muscle cramping. There does need to be a balance between rest and exercise/activity: too little exercise or activity will cause weakness from disuse; too much exercise/activity may cause further weakness and muscle cramping and pain. Therefore, some form of gentle exercise is recommended for most polio patients; however, some patients may be too weak and fatigued to perform any additional exercise. It is best to have an individualized exercise program designed by a physiotherapist to suit your needs and abili-ties, if exercise is appropriate.

3. Assistive Devices

Assistive devices such as canes, crutches, wheelchairs, equipment to aid in activities of daily living, and orthoses (braces) may also be recommended for some individuals. These devices may improve mobility, improve safety, decrease pain, and prevent deformities. Even though these types of devices are obvious reminders of polio and may be difficult to accept, they are important to consider since they can be very helpful.

4. Emotional Impacts

The onset of the late effects of polio affect the physical, the emotional and the psychological well-being of the individual. Adjustments to polio symptoms may affect activities both in the home and in the workplace, social interaction with family and friends and life skills. Each of these situations can be stressful and create anxieties for individuals and families. Past strate-gies of coping with pain, fatigue and weakness, may no longer be helpful and new ways need to be learned. It may be difficult to ask for assistance and many people only do so in a crisis situation.

Others see asking for help and/or using assistive devices as a step toward dependency. Regardless, it is important to understand that by adjusting to ones' limitations, further deterio-ration can be minimized. Meanwhile, independence can be maintained and quality of life ensured.

5. Medications

Some medications are available to treat specific symptoms. Pain from arthritis, bursitis, tendonitis may respond to medications such as acetominephen, aspirin, ibuprofen and other similar medications. New non-steroidal anti-inflammatory medications (similar to ibuprofen) which produce much less gastro-intestinal difficulties are now available. Pain from fibromyalgia (a disorder characterized by chronic, generalized muscle pains) may improve with amitriptyline (Elavil) and other medications/treatments. Fatigue from PPS may improve with pyridostigmine (Mestinon). In addition, new medications may become available for treatment of weakness and fatigue in the next few years. If possible, it is also important to avoid some medications which may increase muscle fatigue/weakness and general fatigue. Some anesthetics should be avoided, and it is important to notify your anesthesiologist prior to surgery that you have had polio.

G. Resources

1. Polio Quebec Association

The Polio Quebec Association was formally founded on October 14th, 1986, to represent the interests of people who have had polio. The Association has many functions. Its objectives are:

To create and maintain a registry in order to compile, publish

and dis-seminate information about polio and available services aimed at improving the quality of life of per-sons with PPS.

To exchange ideas and information with local, provincial,

National and international organizations through newsletters and other media.

To encourage research into the treatment and possible cure of

PPS.

To foster public awareness about polio, PPS and the importance

of preventive measures.

To advocate on behalf of people who had polio.

For more information on the Association, or to become a member, please contact the Polio Quebec Association at:

Association Polio Québec Association

C.P. 745

Succursale Jean-Talon

Montréal, Québec H1S 2Z5

Information

Montreal: (514) 935-9158 (English)

259-2451 / 932-6092 (French)

2. CLSC (Local Community Service Center)

The CLSC provides basic health care services and check-ups from a multi-disciplinary team comprised generally of doctors, nurses, physiotherapists, occupational therapists, and social workers. They also have visiting homemakers who provide help in the household and with personal care. For example, they can offer assistance in cleaning the house and with bathing. They can also help with some shopping, banking, and laundry. Visiting nurse services may also be provided for the patient who is not fully autonomous. For further information on these services, please contact the CLSC in your local area.

3. The March of Dimes

The March of Dimes was founded in 1951 in response to the great polio epidemics of the 1930's and 1940's. Its purpose was to find ways to prevent the disease and to assist in polio rehabili-tation.

Following the discovery of the Salk vaccine in 1955, new cases of polio diminished. The March of Dimes had achieved its objective and polio became a forgotten disease. Approximately 25 years later, new problems affecting the lives of polio survivors appeared. Recognizing and addressing the late effects of polio became a real and valid concern.

The Quebec March of Dimes has been supportive of the Polio Quebec Association, and has provided some funding of the Association and of research in the past.

The Ontario March of Dimes has been very responsive to the needs of post-polio individuals and has sponsored several conferences on the late effects of polio. Audio cassette tapes from these conferences, other educational material and conference proceedings are available. For more information about any of the above, contact their Post- Polio Coordinator at:

Ontario March of Dimes

60 Overlea Blvd

Toronto, Ontario M4H 1A4

Tel: (416) 425- 3463

The international office of the March of Dimes in White Plains, New York, is organizing an international symposium on PPS, which will take place in May 2000.

4. GINI (Gazette International Networking Institute)

The late Mrs. Gini Laurie was the founder of the Rehabilitation Gazette. Published in St. Louis, Missouri, the magazine has over 50,000 readers in 85 countries. This bi-annual journal offers practical advice and equipment tips for people with physical disabilities.

Gini Laurie, a long time advocate for the independent living movement, was among the first to recognize post-polio problems. Letters from her readers discussing their problems and con-cerns prompted her to organize the first international conference on post- polio issues which was held in Chicago in 1981. This organization has organized an international post-polio conference every few years. Since the illness and death of Gini Laurie, Joan Headley has been the Executive Director of the organization. For further information write or call:

G.I.N.I.

4207 Lindell Blvd, Suite 110

St. Louis, MO 63108-2915, USA

Tel: (314) 534-0475

In addition, Gini Laurie published a practical book entitled "Handbook on the Late Effects of Poliomyelitis for Physicians and Survivors". A new edition of this book appeared in 1999. This book can be purchased from GINI at the above address.

This clinic was started by Dr. Neil Cashman, Neurologist. Dr. Daria Trojan, physiatrist, joined Dr. Cashman in 1989 and to this time more than 800 patients have been evaluated. Since Dr. Cashman's departure in 1998, Dr. Trojan has been the Director of the Post-Polio Clinic. The consulting Neurologist for the clinic is now Dr. Daniel Gendron. Our work on PPS and with post-polio patients continues to involve three broad areas: (1) clinical care of post-polio patients; (2) education of health care professionals and lay individuals; (3) clinical research on PPS.

Currently, a first-time clinic appointment usually involves an evaluation by Dr. Trojan. Subsequently, at another appointment, patients may also be evaluated by Dr. Gendron, Neurologist and Director of EMG Laboratory at the MNH. Dr. Gendron may perform electromyography (EMG) studies on some patients. Follow-up visits occur every 6 to 12 months. Some patients are also referred to a pulmonary specialist at the Royal Victoria Hospital (RVH) with a special interest in post-polio problems. Many patients are referred to physiotherapy and occupational therapy of the MNH. Patients can also be referred to orthotists (outside of the hospital) for braces and assistive devices, if necessary. Social service consultations are also available at the hospital, if necessary.

The post-polio clinic provides volunteers for several ongoing clinical studies. The multi-centered, randomized, double-blinded placebo- controlled trial of pyridostigmine (Mestinon) in PPS has been completed. This was the first multi-centered clinical trial in this disorder. The placebo-controlled phase of the trial has been analyzed, and the results published in October 1999. The open-label phase of the study (which consisted of an evaluation 6 months after the completion of the placebo-controlled phase of the trial) is being analyzed. Six centers in Canada and the United States participated in this trial; the primary center was in Montreal. We are also performing other analyses of the data collected during this clinical trial. The goals of these analyses are to determine (1) if serum insulin-like growth factor-I (IGF-I) is associated with various clinical parameters in PPS, such as isometric strength, fatigue and quality of life; (2) the clinical parameters which have the most important impact on quality of life in PPS patients; and (3) the predictive factors and correlates for muscle and joint pain in PPS patients.

Other similar clinical studies which are in progress include another smaller placebo-controlled, discontinuation trial of pyridostigmine in patients who are regularly taking the medication, studies on the electrophysiology and electrodiagnosis of PPS, and Magnetic Resonance Spectroscopy (MRS) studies of the brain in PPS patients (to determine whether or not there is nerve cell loss in certain areas of the brain). A special thank you is extended to those who participated in the studies to help make them possible.

In summary, a range of activities relevant to post-polio patients is currently being conducted at the MNH and MNI, with important contributions from other departments of McGill University, and institutions outside Montreal.

About the site: "The core of this site is a library of well over one hundred full text articles on Post-Polio conditions, many from peer reviewed medical journals. The library is catalogued to assist reading order and new articles are added typically every few weeks. A categorised directory of polio resources is also provided with every entry having a description."

Acknowledgements

This booklet is based on a similar information handbook produced by the West Park Hospital Post-Polio Clinic in Toronto, Ontario. We wish to thank the West Park Hospital Post-Polio Clinic for allowing us to use their handbook. We are indebted to our clinical secretary, Ms. Danielle Lafleur, for her work in our clinic, to Ms. Liliana Cetola for her assistance in the preparation of the manuscript, and to Ms. Josée Lemoignon for translation of the manuscript. The figures are reprinted from: Trojan DA, Cashman NR. Current Trends in Post-Poliomyelitis Syndrome. New York, NY: Milestone Medical Communications, a division of Ruder-Finn, 1996.

IMPORTANT PLEASE READ: Materials provided by the Resource Centre are for

educational purposes only, they are not intended to replace the advice or instruction of a professional healthcare practitioner, or to substitute for medical care. If you have any further questions regarding information you obtained from sources accessed through this service, please contact a qualified healthcare practitioner. Evaluating the merits of health information should always involve the assistance of a trusted healthcare practitioner who is familiar with your health history, and who can bring expertise and experience to bear on your situation. Beware that general information regarding health and disease is not always appropriate when applied to individual cases. Resource Centre personnel are not trained to interpret health information. Information provided by the Resource Centre does not imply endorsement or recommendation by the Montreal Neurological Hospital of the McGill University Health Centre, the Patients' Committee, its affiliates, or individuals.

The Polio Crusade

THE POLIO CRUSADE IN AMERICAN EXPERIENCE A GOOD VIDEO
THE STORY OF THE POLIO CRUSADE pays tribute to a time when Americans banded together to conquer a terrible disease. The medical breakthrough saved countless lives and had a pervasive impact on American philanthropy that ...
Continue reading..http://www.pbs.org/wgbh/americanexperience/polio/

Who we are?

WHO WE ARE

We are a non-profit civil organization legally incorporated in Mexico City, on January 8, 2004. Our main goal is to spread information on Post-Polio Syndrome by all possible means, and to offer help to any person suffering from Poliomyelitis or Post-Polio Syndrome.

Fortunately, thanks to science and medical research, we know the etiology of Post Polio Syndrome and its different pathological manifestations. This knowledge will help persons who had Poliomyelitis to make decisions to avoid or to delay the first’s signs of Post-Polio.

One of the Post-Polio Association Litaff A.C. goals is to provide information onpreventive medicine through medical articles available by free downloads, organization of conferences and seminars,meditation specialized workshops and access to an excellent Cromotherapy treatment for Post-Polio Syndrome and Fibromyalgia, access to nutrition programs, etc.

One of the multiple goals of our association is to help people suffering from Poliomyelitis sequels, Chronic Fatigue or Fibromyalgia

to modify or to improve their life quality.

We base our advice on the experience of specialists and their medical recommendations. One really important goal always present in our minds is to help and support the family and friends of those who suffer from Post-Polio Syndrome, Fibromyalgia and Chronic Fatigue, because they represent a fundamental support for them. We are able to provide them with reliable information and psychological help.

We are concerned about the architectonic barriers in our cities and we’re working every day to improve and make the movements of any person with a motor incapacity easier. In this regard, we do have a good support from our authorities and from the society. Likewise, we offer some good job opportunities with flexible schedules, so every person has a chance to adapt.

We invite every poliomyelitis survivor, every person suffering from Fibromyalgia and/or Chronic Fatigue to join this association through this page, so we can share our experiences and we can also be able to extend the network of families and friends of Post-Polio Litaff A.C. through its chat room, its discussion forum and its conferences. We invite you too, of course, to benefit of all the advantages we offer.

We do consider that this Website could be a valuable media for spreading all the information gathered on Post-Polio Syndrome and, of course, an excellent way of knowing each person affected and helping each other.

This could be done in different ways: direct economical contribution, professional medical attention, or even by donations of wheel chairs, crutches, scooters, and any aid you could think of.

Any doctor who wishes to join us to help our members, any volunteer who wants to help in the activities that Post Polio Litaff A.C.Organizes every day is absolutely welcome.

Of course, we’re aware of the fact that our goals represent an incredible task. However, we do have great enthusiasm and spirit of solidarity that we are putting at the service of this noble cause, inspired by The Supreme Power that makes it All Possible.

As a Post-Polio survivor, I send this message to all those who suffered from Poliomyelitis. I really want to tell you that you are not alone, we’re numerous persons suffering from this Syndrome and, in the same way we fought the Poliomyelitis in our childhood, we’re going to get together and fight together the battle against this still unknown Syndrome. Together, we will force the world to know about it.

In case you’re already a Post-Polio Syndrome victim, the Post-Polio Association Litaff A.C. is here, so that together we can join ours forces and enthusiasm to get the necessary support.

You are all welcome to become part of the association.

“From this day, let’s try to live one day at a time without forcing ourselves to do more than our body is able to” and let’s live with a positive attitude facing forward this event that we can’t modify now. That’s why accepting it with a positive attitude will help any person suffering from it.

History of Polio

History of polio

In the early 20th century, polio was one of the most feared diseases in industrialized countries, paralysing thousands of children every year. Soon after the introduction of effective vaccines in the 1950s and 1960s however, polio was brought under control and practically eliminated as a public health problem in these countries.

It took somewhat longer for polio to be recognized as a major problem in developing countries. Lameness surveys during the 1970s revealed that the disease was also prevalent in developing countries. As a result, during the 1970s routine immunization was introduced worldwide as part of national immunization programmes, helping to control the disease in many developing countries.

In 1988, when the Global Polio Eradication Initiative began, polio paralysed more than 1000 children worldwide every day. Since then, 2.5 billion children have been immunized against polio thanks to the cooperation of more than 200 countries and 20 million volunteers, backed by an international investment of more than US$ 8 billion.

Today, polio has been eliminated from most of the world and only four countries remain endemic. In 2009, fewer than 2000 cases were reported for the entire year.

Use this interactive timeline to trace the history of polio from 1580 B.C. to the present.

A 41-year-old man developed an acute illness at the age of 9 months during which, following a viral illness with headache, he developed severe weakness and wasting of the limbs of the left side. After several months he began to recover, such that he was able to walk at the age of 2 years and later was able to run, although he was never very good at sports. He had stable function until the age of 18 when he began to notice greater than usual difficulty lifting heavy objects. By the age of 25 he was noticing progressive difficulty walking due to weakness of both legs, and he noticed that the right calf had become larger. The symptoms became more noticeable over the course of the next 10 years and ultimately both upper as well as both lower limbs had become noticeably weaker.

On examination there was wasting of the muscles of upper and lower limbs on the left, and massively hypertrophied gastrocnemius, soleus and tensor fascia late on the right. The calf circumference on the right exceeded that on the left by 10 cm (figure1). The right shoulder girdle, triceps, thenar eminence and small muscles of the hand were wasted and there was winging of both scapulae. The right quadriceps was also wasted. The wasted muscles were also weak but the hypertrophied right ankle plantar flexors had normal power. The tendon reflexes were absent in the lower limbs and present in the upper limbs, although the right triceps was reduced. The remainder of the examination was normal.

The patient's legs, showing massive enlargement of the right calf and wasting on the left

Questions

1

What is that nature of the acute illness in infancy?

2

What is the nature of the subsequent deterioration?

3

What investigations should be performed?

4

What is the differential diagnosis of the cause of the progressive calf hypertrophy?

Answers

QUESTION 1

An acute paralytic illness which follows symptoms of a viral infection with or without signs of meningitis is typical of poliomyelitis. Usually caused by one of the three polio viruses, it may also occur following vaccination and following infections with other enteroviruses.1 Other disorders which would cause a similar syndrome but with upper motor neurone signs would include acute vascular lesions, meningoencephalitis and acute disseminated encephalomyelitis.

QUESTION 2

A progressive functional deterioration many years after paralytic poliomyelitis is well known, although its pathogenesis is not fully understood.2 It is a diagnosis of exclusion; a careful search for alternative causes, for example, orthopaedic deformities such as osteoarthritis or worsening scoliosis, superimposed neurological disorders such as entrapment neuropathies or coincidental muscle disease or neuropathy, and general medical causes such as respiratory complications and endocrinopathies.3

QUESTION 3

Investigations revealed normal blood count and erythrocyte sedimentation rate and normal biochemistry apart from a raised creatine kinase at 330 IU/l (normal range 60–120 IU/l), which is commonly seen in cases of ongoing denervation. Electromyography showed evidence of denervation in the right APB and FDI with polyphasic motor units and complex repetitive discharges, no spontaneous activity in the left calf and large polyphasic units in the right calf consistent with chronic partial denervation. Motor and sensory conduction velocities were normal. A lumbar myelogram was normal. Magnetic resonance imaging (MRI) scan of the calves is shown in figure2.

Axial T1 weighted MRI scan (TR 588 ms, TE 15 ms) of the calves, showing gross muscle atrophy and replacement by adipose tissue on the left, and hypertrophy of the muscles on the right, with only minor adipose tissue deposition

QUESTION 4

The differential diagnosis of the progressive calf hypertrophy is given in the box.